The systemic involvement in scleroderma.

A survey was made of the systemic involvement in 38 patients with scleroderma: 33 with the acrosclerotic form (Type 1, 18, Type 2, 15) and five with the diffuse form. The study comprised inquiry about symptoms, physical examination, and the laboratory tests, such as radiological examination of chest and hands, barium swallow and meal X-ray examination, electrocardiography, pulmonary function tests, haematology tests, examination for autoantibodies, and a battery of biochemical tests. Evidence of some systemic involvement (that is, in addition to skin) was almost universal. Similar disturbances occurred both in the acrosclerotic and in diffuse forms. The most common clinical involvement was that of the joints and gastrointestinal tract. The most common confirmatory signs were a positive "neck test" (tethering of the skin of the root of the neck and upper part of the chest on extending the head) and telangiectasia. The most common abnormalities in test results were those found in X-ray films of the hands (about 80%), and in pulmonary function, and barium swallow and meal X-ray studies (each about 70%). The most frequent abnormalities in the biochemical scan were increased levels of immunoglobulin M (IgM), and decreased creatine clearance.